Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics

Small molecules that correct the folding defects and enhance surface localization of the F508del mutation in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) comprise an important therapeutic strategy for cystic fibrosis lung disease. However, compounds that rescue the F508del mutant p...

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Detalles Bibliográficos
Autores principales: Chung, W. Joon, Goeckeler-Fried, Jennifer L., Havasi, Viktoria, Chiang, Annette, Rowe, Steven M., Plyler, Zackery E., Hong, Jeong S., Mazur, Marina, Piazza, Gary A., Keeton, Adam B., White, E. Lucile, Rasmussen, Lynn, Weissman, Allan M., Denny, R. Aldrin, Brodsky, Jeffrey L., Sorscher, Eric J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2016
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5061379/
https://www.ncbi.nlm.nih.gov/pubmed/27732613
http://dx.doi.org/10.1371/journal.pone.0163615

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